Purpose To describe the visual presentation and factors affecting visual outcome in pediatric patients treated for craniopharyngioma at a referral center in the East Coast states of Peninsular Malaysia. Methodology A retrospective review of medical records of children aged 17 years and below who had been treated for craniopharyngioma in Hospital Universiti Sains Malaysia from January 2014 to December 2018. The data collected included age, gender, presenting symptoms and duration, visual acuity, visual fields, color vision, light brightness, relative afferent pupillary defects, fundus examination and cranial nerves examination. The best corrected visual acuity during presentation, and after a one-year post-operative period, was documented. Records on investigations, surgical procedures, therapeutic modalities and recurrences were also reviewed. Results A total of 11 pediatric patients (22 eyes) were recruited. Fifty percent presented with optic atrophy. The mean duration of the onset of symptoms before consultation was 22.3 (24.5) months. A final best corrected visual acuity of 6/12 (20/40) or better was observed in 50% of the patients. There was a statistically significant association between presenting visual acuity, optic nerve function and visual field defects, and the final visual outcome. Conclusions Visual presentations in our study were fairly similar to previous reported studies. One-third presented late with permanent visual loss. Almost half had significant visual impairment after one-year post-operative period. Significant associations were observed between presenting visual acuity, duration of symptoms, impairment of optic nerve function tests, and visual field defects during presentation, and final visual acuity at one year after treatment.
A 19-year-old man presented with sudden onset of right eye ptosis, diplopia and giddiness. He had no previous medical illnesses with negative history of exertional dyspnoea, epistaxis, haemoptysis, palpitations, chest pain and chronic cough. Examination revealed central cyanosis, digital clubbing, polycythaemia, partial ptosis of right eye, diplopia on right gaze and dilated right pupil. Examination of the chest revealed pectus excavatum but no cardiac murmurs were heard. Investigations revealed a solitary right pulmonary arteriovenous malformation with two feeder vessels which were successfully embolized surgically.
Acute lymphoblastic leukemia (ALL) is the most common
childhood leukaemia. It is a malignant neoplasm caused by the proliferation of
poorly differentiated precursors of the lymphoid cells. It is relatively
uncommon in adult. In adult ALL, central nervous system (CNS) involvement
is associated with poor prognosis. The incidence of CNS involvement has
been reported between 7% and 15 %. We report a case of optic nerve
infiltration in ALL in a 49 years old gentleman. He was diagnosed with
precursor-B ALL. He was treated with chemotherapy and CNS prophylactic
regime. He presented with sudden left eye loss of vision for one-day duration
with history of right eye inferior visual field loss for the past three months. His
visual acuity was no perception to light on the left eye and 6/9 on the right
eye. There was marked left relative afferent pupillary defect. The right eye
showed decreased in optic nerve function with inferior visual field defect.
Anterior segment examination was unremarkable in both eyes. Left optic disc
appeared normal but the right optic disc was pale. Blood investigation
showed no sign of infection or haemoconcentration. Cerebral spinal fluids
examination revealed abundant of white cells and blast cells. Magnetic
resonant imaging showed bilateral optic nerve enhancement suggesting of
bilateral optic nerves infiltration. He was started on a new regime of
chemotherapy followed by cranial radiotherapy. Unfortunately, he succumbed
to death due to septicaemia. There are variations in clinical presentation of
optic nerve infiltration in leukaemic patients. Normal appearance of optic disc
may not exclude the possibility of infiltration by malignancy. Assessment of
the optic nerve function and imaging is helpful for the detection of leukaemic
infiltration. Early detection of optic nerve infiltration is important for initiation or
change of therapy to prevent mortality.
A 22-year-old Malay female presented with left eye floaters for 2 weeks, associated with temporal visual field defect and metamorphopsia for 3 days. She has a guinea pig and a hedgehog at home, but denied being bitten or scratched by them. Her visual acuity at presentation was 6/12 on the left eye and 6/6 on the right eye. Her left eye relative afferent pupillary defect was barely positive with mild anterior chamber reaction. Fundus examination of the left eye showed mild vitritis, swollen optic disc with macular star, crops of active choroidal lesions at superonasal retina with a linear arrangement in the form of migratory track nasally. However, there were no nematodes seen on fundus examination. Investigations showed normal full blood count with no eosinophilia and positive serology test for Bartonella henselae. She was diagnosed to have dual infection - diffuse unilateral subacute neuroretinitis (DUSN), based on the presence of crops of choroidal lesions with migratory track, and cat scratch disease (CSD) based on a positive serological test. She was treated with oral albendazole 400 mg 12 hourly for 6 weeks for DUSN and oral doxycycline 100 mg 12 hourly for 4 weeks for CSD. Focal laser had been applied to the area of migratory track in the left eye. Her left eye vision improved to 6/6 at 1 month after treatment, with resolution of neuroretinitis.
We report a case of diffuse unilateral subacute neuroretinitis in a young boy with no clinical visualization of nematode. The diagnosis was made based on clinical findings and detection of Toxocara immunoglobulin G by Western blot test. An 11-year-old Malay boy presented with progressive blurring of vision in the left eye for a duration of 1 year. It was associated with intermittent floaters. Visual acuity in the left eye was 6/45 and improved to 6/24 with pinhole. There was positive relative afferent pupillary defect, impaired color vision, and presence of red desaturation in the left eye. There were occasional cells in the anterior chamber with no conjunctiva injection. Posterior segment examination revealed mild-to-moderate vitritis and generalized pigmentary changes of the retina with attenuated vessels. The optic disk was slightly hyperemic with mild edema. There was presence of multiple, focal, gray-white subretinal lesions at the inferior part of the retina. Full blood picture results showed eosinophilia with detection of Toxocara immunoglobulin G by Western blot test. Investigations for other infective causes and connective tissue diseases were negative. The diagnosis of diffuse unilateral subacute neuroretinitis secondary to Toxocara was made based on clinical findings and laboratory results. He was treated with oral albendazole 400 mg daily for 5 days and oral prednisolone 1 mg/kg with tapering doses over 6 weeks. At 1 month follow-up, the inflammation had reduced, and multiple, focal, gray-white subretinal lesions were resolved; however there was no improvement of vision.